What is CSS Syndrome?

What is CSS Syndrome?

Churg-Strauss syndrome (CSS) or eosinophilic granulomatosis with polyarteritis (EGPA) is a type of vasculitis. These are disorders characterized by inflammation of blood vessels. This inflammation often affects the lungs, skin, nerves and stomach. Asthma is the most common sign of CSS/EGPA.

Is Churg-Strauss syndrome life threatening?

Churg-Strauss syndrome can affect many organs, including the lungs, sinuses, skin, gastrointestinal system, kidneys, muscles, joints and heart. Without treatment, the disease can be fatal.

What is the life expectancy of Churg-Strauss syndrome?

What Is the Life Expectancy for Churg-Strauss Syndrome? In the past, Churg-Strauss syndrome was fatal within months of 70%-90%. This means that 5 years following diagnosis, 70%-90% of patients are still alive. Patients over age 65 have a poorer prognosis.

What is the treatment for CSS?

Treatment usually includes high doses of cortisone-related medication (such as prednisone or prednisolone) to calm the inflammation and suppression of the active immune system with cyclophosphamide (Cytoxan). Traditionally, cyclophosphamide has been given for a year or more in patients with Churg-Strauss syndrome.

Who gets Churg-Strauss?

Anyone can get Churg-Strauss. But things that make it more likely include: Age: On average, people are between 30 and 50 when they get diagnosed. Asthma or nasal problems: Most people who get it have chronic asthma, nasal allergies, or chronic sinusitis.

Can you live a normal life with Churg-Strauss syndrome?

If Churg-Strauss syndrome is caught and treated before any major organ damage has occurred, you can live a fairly normal life. If organ damage has occurred, your future prognosis will be determined by the severity of the damage, and how well it responds to treatment.

How many people are diagnosed with Churg-Strauss syndrome?

EGPA seems to affect men and women equally. Around 11-13 people per million are diagnosed with EGPA. The average age of someone with a new diagnosis is 40 years old, and it is very rare in children or those over 65 to be diagnosed.

Can Churg-Strauss go into remission?

There’s no cure for Churg-Strauss syndrome, also known as eosinophilic granulomatosis with polyangiitis (EGPA). But medications can help manage your symptoms.

How do you diagnose Churg-Strauss?

An X-ray or CT scan looks for damage in the lungs or checks for growths called polyps in the sinuses. Biopsy. If other tests suggest that you might have Churg-Strauss syndrome, your doctor will confirm the results with a biopsy. The doctor removes a small piece of tissue from your lungs, skin, or other organs.

How is CSS an autosomal dominant genetic disorder?

CSS appears to be inherited as an autosomal dominant condition. Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother. Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary to cause a particular disease.

Are there any psychiatric disorders that overlap with CSS?

Psychiatric disorders are common in CSS as well. Research suggests that’s because they all involve dysregulation of the same neurotransmitters, with the dysregulation in CSS in different regions of the brain than in psychiatric disorders. 3  Psychiatric conditions that commonly overlap with CSS include:

Can a person with CSS have heart problems?

Some individuals with CSS may also have heart abnormalities at birth. In addition, a brain abnormality known as Dandy-Walker malformation has been reported in some cases. This condition is characterized by cystic malformation and expansion of one of the cavities in the brain (fourth ventricle).

What is the difference between allergies and CSS?

An illness described as a CSS involves something called central sensitization. “Central” means the central nervous system, which is made up of your brain and spinal cord. “Sensitization” is the end result of something that has made you sensitive. Allergies are the type of sensitivity people are generally…